A Neonatal Case of Septo-Optic Dysplasia with Schizencephaly.

Lee,  Won Hee; Kim,  Eun Young; Park,  Sang Kee

Case Report

Journal of the Korean Society of Neonatology 2009;16(1):81-84.
Published online May 1, 2009.

A Neonatal Case of Septo-Optic Dysplasia with Schizencephaly.

Won Hee Lee, Eun Young Kim, Sang Kee Park

Department of Pediatrics, Collage of Medicine Chosun University, Gwangju, Korea. skpark@chosun.ac.kr

분열뇌증을 동반한 중격 시신경 형성장애 신생아 1례

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Abstract

Septo-optic dysplasia is a rare congenital disorder characterized by the absence of the septum pallucidum, hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. Schizencephaly is an abnormal cleft in the brain, lined with gray matter which may communicate with the ventricular system. Septo-optic dysplasia with schizencephaly is associated with endocrinologic disorders, visual impairment, mental retardation, and seizures. We report a case of septo-optic dysplasia with schizencephaly which was diagnosed in the early neonatal period.

Key Words: Septo-optic dysplasia; Schizencephaly